Simultaneous Testicular and Appendix Testis Torsion: A Novel Clinically Relevant Association.

ABSTRACT: Torsion of the appendix testis (TAT) and testicular torsion (TT) are the most common causes of acute scrotum in the pediatric population. They usually present as separate conditions and have distinct managements. We report a unique case of a 16-year-old boy with concomitant TAT and TT. The role of TAT as a trigger factor to TT is addressed, and its clinical repercussion on the management of acute scrotum is discussed.

Measuring otoplasty outcome: Expanding the validity to caregivers' perspective and to Portuguese-speaking children.

OBJECTIVE: The GBI (Glasgow Benefit Inventory) and the GCBI (Glasgow Children's Benefit Inventory) are used to evaluate patient-reported Health-Related Quality of Life (HRQoL) following otoplasty for prominent ears. The aim of this study was to validate them through patients' and caregivers' perspectives and in Portuguese language versions. STUDY DESIGN: After translation to Portuguese, the GBI and the GCBI were applied to patients (n = 255) who underwent otoplasty throughout a five-year span and to their caregivers, respectively. A second set of questionnaires (retest) was applied to assess reliability. Caregivers were subsequently contacted to evaluate aesthetic satisfaction, recommendation to others, postoperative complications and main motivation for surgery. SETTING: Paediatric Surgery Department of a tertiary hospital in Porto, Portugal. PARTICIPANTS: 134 patients (aged 10.9 ± 3.6 years, 61.2% males) and their caregivers (52.5% responders). MAIN OUTCOME MEASURES: Impact on HRQoL using the Portuguese GBI and GCBI (global score, -100 to 100). Validity assessed by principal component factor analysis, Cronbach's α and Intraclass Correlation Coefficient (ICC). RESULTS: The GBI was 30.1 ± 25.7 and the GCBI was 22.6 ± 23.0. For the GBI, three subscales were identified, explaining 63.3% of variance. For the GCBI, two subscales were identified, accounting for 66.8% of variance. Both the GBI and the GCBI showed a high internal consistency (α = 0.92 and 0.94, respectively). 79% were satisfied and 92% recommended the surgery. Statistically significant higher scores were found in satisfied patients, in those who underwent surgery due to teasing, and in females. CONCLUSIONS: The Portuguese GBI and GCBI proved to be valid and reliable to evaluate HRQoL following otoplasty in Portuguese-speaking children. The outcome was very favourable; almost all patients would recommend the surgery.

Trans-umbilical laparoscopic-assisted appendectomy in the pediatric population: comparing single-incision and 2-trocar techniques.

INTRODUCTION: Trans-umbilical laparoscopic-assisted appendectomy (TULAA) combines advantages of open surgery with benefits of laparoscopy and can be performed either by single-incision or with two trocars; the present study aims to compare outcomes and cost of these techniques in a pediatric serie. MATERIAL AND METHODS: Patients undergoing TULAA between July 2013 and June 2014 were retrospectively reviewed. There were two groups: A (single-incision) - one umbilical trocar for a telescope with inbuilt working channel; and B (2-trocar) - one umbilical trocar for a 'standard' telescope plus a supra-pubic trocar for instruments. RESULTS: One-hundred and eighty-nine patients (112 males, age: 12[2-17] years) underwent TULAA; 32(17%) presented with perforated appendicitis. Group A encompassed 157 and group B 32 patients; age, gender, proportion of perforated appendicitis, and follow-up were similar. Operative time was 46 [21-145] min in group A, and 54.5[30-111] in group B (A vs B, p = 0.087). Length of hospital stay was 2 [1;22] days in group B, and 2 [0;24] in group A (A vs B, p = 0.136). The prevalence of post-operative complications was similar: 11 (7%) cases in group A and 2 (6.3%) intra-abdominal abscesses in group B. Using disposable trocars, the more expensive starting point of single-incision technique may be reversed after 27 procedures. CONCLUSION: Single-incision and 2-trocar TULAA are effective techniques with low complication rates and similar outcomes. Despite being technically more demanding, single incision may be advantageous because there are no visible scars, and potentially cheaper in hospitals where disposable trocars are used.

Fascicular Phrenic Nerve Neurotization for Restoring Physiological Motion in a Congenital Diaphragmatic Hernia Reconstruction With a Reverse Innervated Latissimus Dorsi Muscle Flap.

Congenital diaphragmatic hernia is a severe developmental anomaly characterized by the malformation of the diaphragm. An innervated reversed latissimus dorsi flap reconstruction for recurrent congenital diaphragmatic hernia has been described as an alternative to prosthetic patch repair to achieve pleuroperitoneal separation. However, there is very little supporting scientific data; therefore, there is no real basic understanding of the condition of the phrenic nerve in the absence of diaphragmatic muscle or even the neurotization options for restoring neodiaphragmatic muscle motion. We have reviewed the literature regarding phrenic nerve anatomy and neurotization options, and to our knowledge, this is the first time that the application of a fascicular repair is being described where the continuity of one remaining fascicle of the diaphragm has been preserved close to the phrenic nerve distal division. The procedure was undertaken in a 3 year-old boy, with the diagnosis of congenital large posteromedial diaphragmatic hernia and dependence of mechanical ventilation in consequence of severe bronchopulmonary dysplasia.The phrenic nerve divides itself into several terminal branches, usually three, at the diaphragm level, or just above it. This allows the selective coaptation of separate fascicular branches. In the case described, videofluoroscopy evaluation showed no evidence of paradoxical neodiaphragmatic motion, with synchronous contraction movements and intact pleura-peritoneal separation. The child is now asymptomatic and shows improvement of his previous restrictive pulmonary disease.We believe that fascicular repair can achieve some reinnervation of the flap without jeopardizing the potential of diaphragmatic function by contraction of reminiscent native diaphragm.

Abnormal Sonic hedgehog signaling in the lung of rats with esophageal atresia induced by adriamycin.

BACKGROUND: Abnormal lung development was recently described in the rat model of esophageal atresia and tracheoesophageal fistula (EA-TEF). Since in this condition the ventral-to-dorsal switch of Shh expression in the foregut is disturbed, the present study tested the hypothesis that this abnormal expression at the emergence of the tracheobronchial bud might be translated into the developing lung. METHODS: Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle from E7 to E9. Three groups were studied: control and adriamycin-exposed with and without EA-TEF. Embryos were recovered and the lungs were harvested and processed for reverse transcription polymerase chain reaction and immunofluorescence analysis of the Shh signaling cascade. RESULTS: Shh signaling was downregulated at the late embryonic stage of lung development (E13) in embryos with EA-TEF. Throughout the subsequent stages of development, the expression of both Shh and its downstream components increased significantly and remained upregulated throughout gestation. Immunofluorescent localization was consistent with these findings. CONCLUSION: Defective Shh signaling environment in the foregut is present beyond the emergence of lung buds and probably impairs lung development. Later in gestation, lungs exhibited a remarkable ability to upregulate the Shh cascade, suggesting a compensatory response. These findings may be relevant to understand pulmonary disease suffered by children with EA-TEF.

Maternal hyperthyroidism increases the prevalence of foregut atresias in fetal rats exposed to adriamycin.

PURPOSE: Gastrointestinal malformations such as esophageal atresia with tracheoesophageal fistula (EA/TEF) and duodenal atresia (DA) have been reported in infants born to hyperthyroid mothers or with congenital hypothyroidism. The present study aimed to test whether maternal thyroid status during embryonic foregut division has any influence on the prevalence of EA/TEF and DA in an accepted rat model of these malformations. METHODS: Pregnant rats received either vehicle or 1.75 mg/kg i.p. adriamycin on gestational days 7, 8 and 9. Transient maternal hyper or hypothyroidism was induced by oral administration of levothyroxine (LT4, 50 µg/kg/day) or propylthiouracil (PTU, 2 mg/kg/day), respectively, on days 7 to 12 of gestation. Plasma cholesterol, total T3, free T4 and TSH were measured at gestational days 7, 12, and 21. At the end of gestation, the mothers were sacrificed and embryo-fetal mortality was recorded. Fetuses were dissected to determine the prevalence of esophageal and intestinal atresias. RESULTS: At gestational day 12, mothers treated with LT4 or PTU had hyper or hypothyroid status, respectively; plasma cholesterol levels were similar. In the adriamycin-exposed fetuses from hyperthyroid mothers, the embryonal resorption rate and the prevalence of both EA/TEF and DA were significantly higher than in the other groups; maternal hypothyroidism during the same period did not have significant effect on the prevalence of atresias. CONCLUSIONS: Maternal hyperthyroidism during the embryonic window corresponding to foregut cleavage increased the prevalence of both EA/TEF and duodenal atresia in fetal rats exposed to adriamycin. This suggests that maternal thyroid hormone status might be involved in the pathogenesis of foregut atresias and invites further research on this likely clinically relevant issue in humans.

Abnormal control of lung branching in experimental esophageal atresia.

PURPOSE: Esophageal atresia and tracheo-esophageal fistula (EA-TEF) result from abnormal division of the foregut into esophagus and trachea thus, it may influence airway branching and lung development. The present study examined lung morphogenesis in fetuses with EA-TEF focusing in the expression of FGF10 and its receptor FGFR2 IIIb. METHODS: Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle on E7, E8 and E9. Embryos were recovered at E15, E18 and E21 and lungs processed for immunohistochemistry and RT-PCR. Three groups were studied: control, adriamycin-exposed with EA-TEF, and adriamycin-exposed without EA-TEF. Comparisons were performed with Mann-Whitney or t tests (significance level, 5 %). RESULTS: Lung weight at E15 and E18 were significantly lower in adriaEA fetuses in which the relative mRNA levels of FGF10 were significantly higher. These differences disappeared near term. The receptor FGFR2 IIIb messenger was only significantly increased in adria noEA fetuses at E15. Immunohistochemical study was consistent with these findings. CONCLUSIONS: Abnormal expression of FGF10 during earlier stages of development, when the lungs are smaller than controls, suggests a compensatory response aimed at "catching up" delayed tracheobronchial branching. Whether similar changes take place in the human condition and influence respiratory physiology remain to be determined.

Minimally invasive surgery for pilonidal disease: Outcomes of the Gips technique-A systematic review and meta-analysis.

BACKGROUND: Pilonidal disease is a common inflammatory condition that significantly impacts quality of life. Currently, there is a tendency to favor minimally invasive procedures. The present review aims to summarize the evidence and assess the outcomes of the Gips procedure. METHODS: A systematic review was conducted on MEDLINE/Pubmed, Scopus, Web of Science, and Cochrane Library databases until December 2022. Eligible studies included patients with pilonidal disease submitted to the Gips procedure, reporting at least 1 of the following outcomes: wound complications, wound healing time, time to resume daily activities, and recurrence (International Prospective Register of Systematic Reviews protocol: CRD42023389269). The National Institutes of Health assessment tool was used for risk of bias evaluation. Meta-analysis was performed using OpenMeta[Analyst] and R software, and a subgroup analysis was performed when applicable. RESULTS: Thirteen observational studies with a total of 4,286 patients submitted to Gips were included. The pooled wound complications rate was 7.8% (95% confidence interval: 5.1-10.6), the median time to resume daily activities was 1 day (95% confidence interval: 1-2), and the mean wound healing time was 4.7 weeks (95% confidence interval: 3.0-6.4). Subgroup analysis showed that pooled recurrence rate was 6.5% (95% confidence interval: 5.2-7.8) up to 2 years and 38.9% (95% confidence interval: 27.1-50.7) after more than 2 years of surgery. Most results showed substantial heterogeneity across studies. CONCLUSION: Despite apparent favorable outcomes of the Gips procedure, there is a high recurrence rate over time. Because included studies had an observational nature and unstandardized methodologies, comparative randomized controlled trials with longer follow-ups are needed for high-level evidence regarding these outcomes.

Serial transverse enteroplasty (STEP): intermediate outcomes in children with short bowel syndrome.

Short bowel syndrome is the most common cause of intestinal failure in children. The treatment is based on a multidisciplinary approach involving pediatricians, pediatric surgeons, and nutritionists. Surgical procedures for intestinal lengthening may be decisive, having been revalued after the recent description of serial transverse enteroplasty (STEP). We reviewed the patients who underwent the STEP operation for short bowel syndrome in our hospital in order to evaluate medium-term outcome. Between April 2006 and December 2008, three children were submitted to STEP without postoperative complications directly related to the procedure. In two cases the autonomy for oral/enteric feeding was obtained within 3 and 7 months after surgery with sustained growth, persisting at 5 years of follow-up after STEP. One child remained dependent of parenteral nutrition and was submitted to intestinal transplantation 30 months after STEP. However, since STEP until transplantation, it was possible to increase enteric volume and decrease intestinal dilation and the frequency of occlusive episodes. STEP is an effective and safe technique for intestinal lengthening that may allow increased tolerance to oral/enteric feeding or at least alleviate some complications of short bowel syndrome.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center.

OBJECTIVE: To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. METHODS: This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. RESULTS: There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p = 0.033), were born earlier (36 versus 37 weeks, p = 0.006), had lower birth weight (2365 ± 430.4 versus 2944.2 ± 571.9 g; p = 0.001), and had a longer hospital stay (24 versus 9 days, p = 0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). CONCLUSION: Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

OBJETIVO: Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. MéTODOS: Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. RESULTADOS: Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p = 0.033), nasceram mais precocemente (36 versus 37 semanas, p = 0.006), com menor peso ao nascimento (2,365 ± 430.4 versus 2,944.2 ± 571.9 g; p = 0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p = 0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante um tempo mediano de seguimento de 24 meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). CONCLUSãO: Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrer morbidade substancial no médio prazo.

Gastric emptying and antireflux surgery.

PURPOSE: Absence of consistent data on the outcome of gastric emptying after fundoplication raises concerns about preoperative workup and surgical management. This study assessed how gastric emptying evolves after isolated fundoplication in order to determine whether a preoperative investigation and/or a concurrent gastric drainage procedure are justified. METHODS: Eleven children with GERD underwent both pre- and post-operative gastric emptying scintigraphy. No gastric drainage procedures were added to fundoplication. Scintigraphy was performed according to a standardized protocol (labeled meal, 300 ml + 500 µCi (99m)Tc/m(2) bsa). Half-emptying time (t (1/2)), emptying rate and fractions of retention are expressed as the median followed by the [range]; statistical significance level was set at 5%. RESULTS: Postoperative t (1/2) (min) showed a much smaller dispersion and was significantly lower than preoperative one (76 [56;101] vs. 107 [57;186], p = 0.04) with a variation of -48 [-105;19]; there was no individual correlation between pre- and post-operative measurements (r = 0.25). The proportion of patients with delayed gastric emptying (t (1/2) > 100) was significantly lower in the postoperative scintigraphy (9 vs. 55%, p = 0.03). The postoperative emptying rate (%/h) was significantly faster (35.1 [18.9;41.7] vs. 28.5 [16.3;38.7], p = 0.05). The retentions (%) at 30 and 60 min were similar; at 90 and 120 min were significantly lower after surgery (45 [35;54] and 32 [24;46] vs. 53 [33;78] and 41 [25;66], p = 0.03 and 0.05). CONCLUSION: Gastric emptying accelerates significantly after fundoplication with a shift to normal values in the vast majority of patients. Scintigraphy does not predict the postoperative outcome. Therefore, concomitant drainage procedures seem unnecessary and preoperative gastric emptying study not useful.

Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature.

Herlyn-Werner-Wunderlich syndrome, defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian malformation, usually diagnosed after menarche, when symptoms related to hematocolpos arise. Rarely, this malformation is diagnosed in the neonatal period, normally following prenatal diagnosis of renal agenesis. Herein, a case recognized on prenatal imagiology that underwent surgery on the fourth day of life is reported. The records of prepubertal cases were also collected, addressing the clinical and imagiological features. In the presence of a solitary kidney and/or a pelvic mass on prenatal ultrasound, Herlyn-Werner-Wunderlich syndrome should be considered, enabling neonatal treatment.

Biliary Atresia - Clinical Series.

INTRODUCTION: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. MATERIAL AND METHODS: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status. RESULTS: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively. DISCUSSION: The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis. CONCLUSION: In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.

INTRODUÇÃO: A atresia das vias biliares representa a principal causa de morte por insuficiência hepática e a principal indicação para transplante hepático em idade pediátrica. O objetivo deste estudo é analisar a população com este diagnóstico, tratada entre 2000 e 2015 no Hospital de São João. MATERIAL E MÉTODOS: Estudo descritivo, observacional e retrospetivo, incluindo-se doentes com atresia das vias biliares diagnosticados e tratados entre 1 de Janeiro de 2000 e 31 de Dezembro de 2015. Foram analisados dados epidemiológicos, clínicos, bioquímicos, imagiológicos, idade à data da cirurgia, complicações ocorridas e o estado atual. RESULTADOS: Foram avaliados 18 doentes. A idade mediana à data da portoenterostomia de Kasai foi de 63 dias de vida, verificando-se um melhor prognóstico nos pacientes operados antes dos 72 dias. Foi constatada drenagem biliar adequada em 2/3 dos casos aos 6 meses. Verificou-se uma associação significativa entre a ocorrência de episódios recorrentes de colangite e a sobrevida. Registaram-se 5 transplantes e 2 óbitos, um dos quais pós-transplante. A sobrevida com fígado nativo foi de 77.8%, 72.2% e 64.2% ao 1º, 5º e 10º anos de seguimento, respetivamente. DISCUSSÃO: A apresentação e evolução dos pacientes coincidiu, de um modo geral, com o descrito na literatura. No entanto, constatou-se uma taxa de sucesso operatório e de sobrevida com fígado nativo aos 5 e 10 anos superiores à maioria das séries. A idade à data de cirurgia e a recorrência dos episódios de colangite foram os únicos fatores que se mostraram significativamente relacionados com o prognóstico. CONCLUSÃO: Apesar do número reduzido de doentes (1,125/ano), foram registados resultados comparáveis a outros centros de referência.

BOTOX-A injection of salivary glands for drooling.

BACKGROUND AND PURPOSE: Drooling is a challenging entity to manage. Botulinum toxin A (BOTOX-A) infiltration of salivary glands is a promising alternative to surgical treatment. This study aims to assess the outcome of BOTOX-A salivary glands infiltration in children with drooling. METHODS: Patients treated between January 2012 and March 2015 were enrolled. BOTOX-A was injected in the parotid and submandibular glands under ultrasound control and general inhalational anesthesia. The outcome was evaluated through the DSFS: Drooling Severity (1-best to 5-worst) and Frequency (1 to 4) Scale, that was applied before treatment, and 1-, 3-, and 6-month after injection. The inclusion criteria were a DSS ≥4 and/or DFS ≥3. Statistical significance was set at 5%. RESULTS: There were 17 patients aged 12.1±5.1 [4-19]years, all of them with neurologic impairment. After the first injection, 13 (76.5%) patients had reduction of the severity (S) and 12 (70.6%) of the frequency (F) scale; in 6 (35.5%) patients drooling resolved completely. Pre-treatment S+F score was 8.59±0.71 [7-9]; it decreased significantly to 4.65±2.32 (p=0.001) at 1-month post-injection evaluation. At 3-month and 6-month the scores were also significantly lower than the pre-treatment one (4.00±1.96, p=0.002; 5.36±2.20, p=0.005; respectively), but there was a significant increase between the 3-month and 6-month evaluations (p=0.01). With a follow-up of 20.1±9.2 [4-38] months, 4 out of the 13 successful injections needed a second one after 7.5±3.1 [3-10] months. The patient with the longest time not requiring re-injection had 28months of follow-up. One (6%) patient presented mild dysphagia that regressed spontaneously. All but two (88%) parents/caregivers would repeat the treatment. CONCLUSIONS: BOTOX-A seems to be an effective minimal invasive treatment for drooling with few complications. After 6months the need for re-injection becomes substantial but it may not be necessary for several months. Further studies are needed to establish the most effective dosage and frequency of injections. LEVEL OF EVIDENCE: IV.

Short and Medium-term Outcomes of Omphalocele and Gastroschisis: A Survey from a Tertiary Center / Resultados a curto e médio prazo do onfalocelo e gastrosquisis: uma investigação de um centro terciário

Abstract Objective To characterize and compare the outcomes of omphalocele and gastroschisis from birth to 2 years of follow-up in a recent cohort at a tertiary center. Methods This is a retrospective clinical record review of all patients with gastroschisis and omphalocele admitted to the Neonatal Intensive Care Unit between January 2009 and December 2019. Results There were 38 patients, 13 of whom had omphalocele, and 25 of whom had gastroschisis. Associated anomalies were present in 6 patients (46.2%) with omphalocele and in 10 (41.7%) patients with gastroschisis. Compared with patients with omphalocele, those with gastroschisis had younger mothers (24.7 versus 29.6 years; p=0.033), were born earlier (36 versus 37 weeks, p=0.006), had lower birth weight (2365±430.4 versus 2944.2±571.9 g; p=0.001), and had a longer hospital stay (24 versus 9 days, p=0.001). The neonatal survival rate was 92.3% for omphalocele and 91.7% for gastroschisis. Thirty-four patients were followed-up over a median of 24 months; 13 patients with gastroschisis (59.1%) and 8 patients with omphalocele (66.7%) had at least one adverse event, mainly umbilical hernia (27.3% vs 41.7%), intestinal obstruction (31.8% vs 8.3%), or additional surgical interventions (27.3% vs 33.3%). Conclusion Despite the high proportion of prematurity, low birth weight, and protracted recovery, gastroschisis and omphalocele (without chromosomal abnormalities) may achieve very high survival rates; on the other hand, complications may develop in the first years of life. Thus, a very positive perspective in terms of survival should be transmitted to future parents, but they should also be informed that substantial morbidity may occur in the medium term.

Resumo Objetivo Caracterizar e comparar os desfechos do onfalocelo e gastrosquisis desde o nascimento até aos 2 anos de seguimento numa coorte recente de um centro terciário. Métodos Este é um estudo retrospectivo em que foi feita uma revisão dos registos clínicos de todos os pacientes com gastrosquisis e onfalocelo que foram internados na unidade de cuidados intensivos neonatais, entre janeiro de 2009 e dezembro de 2019. Resultados Identificamos 38 pacientes, 13 dos quais tinham onfalocelo e 25 dos quais tinham gastrosquisis. Anomalias associadas estavam presentes em 6 pacientes (46.2%) com onfalocelo e 10 (41.7%) com gastrosquisis. Comparativamente com os pacientes com onfalocelo, os pacientes com gastrosquisis tinham mães mais jovens (24.7 versus 29.6 anos; p=0.033), nasceram mais precocemente (36 versus 37 semanas, p=0.006), com menor peso ao nascimento (2,365±430.4 versus 2,944.2±571.9 g; p=0.001), e o internamento teve uma duração mais longa (24 versus 9 dias, p=0.001). A taxa de sobrevivência neonatal foi de 92.3% para o onfalocelo e 91.7% para a gastrosquisis. Trinta e quatro pacientes foram seguidos durante umtempo mediano de seguimento de 24meses: 13 com gastrosquisis (59.1%) e 8 com onfalocelo (66.7%) apresentaram pelo menos um evento adverso, sobretudo hérnia umbilical (27.3% vs 41.7%), obstrução intestinal (31.8% vs 8.3%) ou intervenções cirúrgicas adicionais (27.3% vs 33.3%). Conclusão Apesar da alta proporção de prematuridade, de baixo peso e de recuperação lenta, os gastrosquisis, assim como os onfalocelos (sem anomalias cromossómicas), podem ter uma taxa de sobrevivência muito alta; por outro lado, nos primeiros anos de vida, podem surgir complicações não desprezíveis. Assim, aos futuros pais pode ser transmitida uma perspectiva muito positiva em termos de sobrevivência, embora eles também devam ser informados de que pode ocorrermorbidade substancial no médio prazo.

Complete Ureteral Duplication: Outcome of Different Surgical Approaches.

INTRODUCTION: The surgical management of complete ureteral duplication anomalies is not consensual. OBJECTIVE: To characterize the pediatric population who underwent surgery for complete ureteral duplication and assess the outcomes of different approaches. MATERIAL AND METHODS: Clinical records from patients treated between January 2008 and June 2014 were retrospectively reviewed. Epidemiology, diagnosis, clinical manifestations and surgical procedures were collected and analysed. Ureteral units were divided into two groups: A, with ureterocele; and B, without it. RESULTS: Forty-one ureteral units from 32 patients with complete duplication underwent surgery. In group A (n = 18), the selected primary procedure was: ureterocele punction (12); ureter reimplantation (3); pyelopyelostomy (2); heminephrectomy (1). A reintervention was required in 3 of the 12 units submitted to punction: heminephrectomy (1), ureteroureterostomy (1), and ureteric reimplantation (1). In group B (n = 23), STING was performed in 10 units, ureteric reimplantation in 3, pyelopyelostomy in 3, ureteroureterostomy in 1, and heminephrectomy in 6; two cases required reintervention. DISCUSSION: A conservative primary approach was favoured in cases with ureterocele and/or reflux in hemisystems worth preserving (53.7%); it was effective per se in 75% (n = 9/12) units in group A and 80% (n = 8/10) in group B. An ablative primary procedure was adopted in 17% (n = 7/41) cases, 5.6% of group A (n = 1/18) and 26.1% of group B (n = 6/23). CONCLUSIONS: A conservative approach is effective as a primary and isolated procedure in the majority of cases with ureterocele or vesicoureteral reflux. Further studies are needed to establish the advantages over primary invasive or ablative approaches.

Introdução: O tratamento cirúrgico das duplicações ureterais completas não é consensual. Objetivos: Caraterizar a população pediátrica submetida a cirurgia para tratamento de duplicações ureterais completas e avaliar resultados de diferentes abordagens. Material e Métodos: Processos clínicos de doentes tratados entre janeiro de 2008 e junho de 2014 foram retrospetivamente revistos. Dados acerca de epidemiologia, diagnóstico, manifestações clínicas e procedimentos cirúrgicos foram recolhidos. As unidades ureterais foram divididas em dois grupos: A, com ureterocelo; e B, sem ureterocelo. Resultados: Quarenta e uma unidades ureterais de 32 doentes com duplicação completa foram intervencionados. No grupo A (n = 18), o procedimento primário selecionado foi: punção de ureterocelo (12); reimplantação de ureter (3); pielopielostomia (2) e heminefrectomia (1). Foi necessário reintervir em três dos 12 casos submetidos a punção: heminefrectomia (1), ureteroureterostomia (1) e reimplantação (1). No grupo B (n = 23), foi efetuado STING em 10 unidades, reimplantação ureteral em três, pielopielostomia em três, ureteroureterostomia em um, e heminefrectomia em seis; dois casos necessitaram de reintervenção. Discussão: Foi favorecida uma abordagem primária conservadora para tratamento de ureterocelo ou refluxo em hemissistemas a preservar (53,7%; n = 22/41), tendo sido eficaz per se em 75% (n = 9/12) unidades do grupo A e 80% (n = 8/10) do grupo B. Uma abordagem ablativa primária foi adotada em 17% (n = 7/14) casos, 5,6% do grupo A (n = 1/18) e 26,1% do grupo B (n = 6/23). Conclusão: Uma abordagem conservadora é eficaz como procedimento primário isolado na maioria dos casos com ureterocelo ou refluxo. Mais estudos são necessários para estabelecer as suas vantagens sobre abordagens primárias invasivas ou ablativas.